Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Prevention |
RCV000721867 | SCV000853016 | likely pathogenic | not provided | 2016-04-20 | criteria provided, single submitter | clinical testing | |
Provincial Medical Genetics Program of British Columbia, |
RCV002067078 | SCV002320868 | likely pathogenic | Cerebral cavernous malformation | 2022-01-01 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002067078 | SCV004294489 | pathogenic | Cerebral cavernous malformation | 2023-10-05 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln643*) in the KRIT1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in KRIT1 are known to be pathogenic (PMID: 10508515, 11222804, 12404106, 24689081). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with cerebral cavernous malformations (PMID: 27790124, 34558799). ClinVar contains an entry for this variant (Variation ID: 590726). For these reasons, this variant has been classified as Pathogenic. |